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Original Investigation
June 4, 2018

Dynamic Changes in White Matter Hyperintensities in Reversible Cerebral Vasoconstriction Syndrome

Author Affiliations
  • 1Institute of Clinical Medicine, National Yang-Ming University School of Medicine, Taipei, Taiwan
  • 2Department of Neurology, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
  • 3Division of Translational Research, Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan
  • 4Faculty of Medicine, National Yang-Ming University, Taipei, Taiwan
  • 5Brain Research Center, National Yang-Ming University, Taipei, Taiwan
  • 6Institute of Neuroscience, National Yang-Ming University, Taipei, Taiwan
  • 7Institute of Biomedical Engineering, National Taiwan University, Taipei, Taiwan
  • 8Department of Radiology, Taipei Veterans General Hospital, Taipei, Taiwan
  • 9Department of Biomedical Imaging and Radiological Sciences, National Yang-Ming University, Taipei, Taiwan
  • 10Institute of Brain Science, National Yang-Ming University, Taipei, Taiwan
JAMA Neurol. Published online June 4, 2018. doi:10.1001/jamaneurol.2018.1321
Key Points

Question  What are the dynamics and pathogenesis of white matter hyperintensities in patients with reversible cerebral vasoconstriction syndrome?

Findings  In this cohort study including 65 patients, white matter hyperintensity loads peaked in the third week postonset and fell to a minimum in the fourth week, which correlated strongly with the severity of vasoconstriction and carotid pulsatility index.

Meaning  The dynamic white matter hyperintensities in patients with reversible cerebral vasoconstriction syndrome may be attributed to regional hypoperfusion and transmission of high pulsatile flow to microcirculation.

Abstract

Importance  White matter hyperintense lesions (WMHs) are highly prevalent in patients with reversible cerebral vasoconstriction syndrome (RCVS); however, their characteristics and underlying pathophysiology are unclear.

Objective  To investigate the spatiotemporal distribution and pathomechanisms of WMHs in patients with RCVS.

Design, Setting, and Participants  We prospectively recruited patients with RCVS over a 3-year period from January 2010 through December 2012 from the headache center or emergency department of Taipei Veterans General Hospital, Taipei, Taiwan, a 2947-bed national medical center. In total, 85 patients with RCVS were approached, of whom 4 declined to participate, 5 declined follow-up scans, 6 were lost to follow-up, and 5 had suboptimal images. Patients received serial isotropic 3-dimension fluid-attenuated inversion recovery sequence imaging (1-mm slice thickness) with a 3-T magnetic resonance imaging machine as well as transcranial and extracranial color-coded sonography on registration and during follow-ups (at 1 and 2 months, with variations adapting to clinical condition). Data were analyzed from January 2015 to May 2017.

Main Outcomes and Measures  The fluid-attenuated inversion recovery lesion segmentation toolbox was used to segment WMHs automatically. The WMHs were classified as periventricular or deep and were segmented into 13 anatomical locations. The neuroimaging scientists who executed the program were blinded to clinical information. Vascular parameters, including the Lindegaard index (vasoconstriction severity), pulsatility index, and resistance index of the internal carotid artery, were independently collected for comparison.

Results  Sixty-five patients with RCVS completed the study and underwent a total of 162 magnetic resonance imaging examinations. Of the 65 included patients, 58 (89%) were women, and the mean (SD) age was 50.1 (8.9) years. The total mean (SD) WMH load peaked at 3.2 (4.4) cm3 in the third week postonset and fell to 0.8 (0.6) cm3 in the fourth week. White matter hyperintensities were predominantly frontal and periventricular. White matter hyperintensity load correlated strongly with Lindegaard index during the second week of the disease course (r = 0.908; P < .001) and also correlated with the pulsatility index and resistance index of the internal carotid artery.

Conclusions and Relevance  White matter hyperintensities in patients with RCVS have a dynamic temporal evolution that parallels disease severity. The finding of partially reversible WMHs deserves attention and should be known by clinicians taking care of patients with RCVS. White matter hyperintensities in RCVS may be attributed, at least partially, to regional hypoperfusion and impaired dampening capacity to central pulsatile flow.

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